Hematologic Changes in α-Thalassemia

Alpha-thalassemia is very common in Thailand. Interaction of the different types of alpha-thalassemia can lead to many alpha-thalassemia syndromes. In this study the authors compare the hematologic data of subjects with various alpha-thalassemia phenotypes. Designation of the genotypes was based on family study and DNA mapping. The results show that there are equivocal hematologic findings among those who have similar molecular defects, i.e., alpha-thalassemia-2 and hemoglobin (Hb) Constant Spring heterozygotes: alpha-thalassemia 1, homozygous alpha-thalassemia 2, and alpha-thalassemia 2/Hb Constant Spring. The severity of these alpha-thalassemia syndromes correlates with the alpha-globin gene expression calculated from the finding of Liebhaber (Liebhaber SA, et al. J Biol Chem 1986; 261:15327-15333).